Implantable devices in young patients: Hitting the reset button on risk versus benefit

Abstract

Inherited arrhythmia syndromes (IAS) such as arrhythmogenic right ventricular cardiomyopathy (ARVC), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), long QT syndrome (LQTS), and short QT syndrome (SQTS) are the major causes of sudden cardiac death (SCD) in young patients. Up to 1 in 300 individuals may be affected by one of these conditions, and when measured in terms of life years lost, only all cancers exceed SCD. It would thus seem intuitive that the implantation of implantable cardioverter-defibrillator (ICDs) in patients with IAS would work to reduce the burden of life years lost due to SCD. In contrast, ICD therapy in young patients has a significantly higher complication rate that needs to be balanced against its perceived benefit. The late Dr Grace Wolff and colleagues described the use of ICDs in pediatric cardiomyopathy as early as 1990. By 1993, the Pediatric Electrophysiology Society described the experience of 177 young survivors of SCD receiving ICDs and suggested that the “use of ICDs in pediatric patients, with implant selection criteria similar to adults, appears valid,” yet little data on device complications was provided. Kron et al reported 5 major complications (including erosion, dislodgment, or infection) in 17 young patients with transvenous ICD over 7.9 months of follow-up (44% per year). Link et al reported more frequent system infections and lead revisions due to malfunction or dislodgment in 11 young patients compared with 309 adults with predominantly (78%) transvenous systems. All these studies preceded the use of ICDs as primary prophylaxis. In a study of both primary and secondary ICD prophylaxis in children (with appropriate therapy rates of 5% per year and 22% per year, respectively), 19% received inappropriate therapy and 25% requiring system replacement for lead or device malfunctions or infection over 3 years (6.3% per year and 8.3% per year, respectively). Alexander et al identified a